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Pancreatic cysts are an uncommon extrarenal clinical feature of autosomal dominant polycystic kidney disease (ADPKD). The prevalence of pancreatic cysts, sonographically assessed in ADPKD and in the different typs of ADPKD (PKD1 and PKD2) has not been reported. We have studied 173 ADPKD patients and 160 non-affected family members and found a prevalence of pancreatic cysts, of 9% in ADPKD patients over 30 years of age. The presence of pancreatic cysts was related to the increasing age, to the female sex and to the type of ADPKD, found exclusively in PKD1 patients. No complications related to pancreatic cysts were recorded. Pancreatic cysts are an unusual feature of ADPKD and do not appear to contribute to morbidity or mortality.

Although cases of autosomal dominant polycystic kidney disease (ADPKD) associated with abdominal aortic aneurysm have been repeatedly reported in the literature, no systematic studies of the aortas of these patients have been performed. In the study presented here, a sonographic study of the abdominal aorta in 139 ADPKD patients and in 149 healthy family members was carried out. For both groups, an increase in aortic diameter related to age and sex, (being wider in men than women) was found. In ADPKD patients, neither a wider aortic diameter nor a higher prevalence of abdominal aortic aneurysms could be found in any age group. It was concluded that, although these patients are prone to develop aortic aneurysms because of hypertension and associated connective tissue disorders, the presence of abdominal aortic aneurysms should be questioned as a frequent feature of ADPKD.

Linkage analysis was performed on 49 Catalan families with autosomal dominant polycystic kidney disease obtained via the Nephrology Department and related nephrology centers. A total of 336 subjects, 267 at risk for the disease, were investigated using three microsatellites linked to polycystic kidney disease Type 1 (PKD1) and three microsatellites linked to PKD2. All of the subjects underwent physical and sonographic examination. The results demonstrate locus heterogeneity, with 0.85 as the maximum likelihood for the proportion of families linked to PKD1. All of the remaining families were found to be linked to PKD2. Analysis of clinical data in the PKD1 group (N = 146) versus the PKD2 group (N = 20) showed a milder form of the disease in the latter, with a later age at diagnosis (27.4 versus 41.4 yr, P = 0.0002), later age of onset of ESRD (53.4 versus 72.7 yr, P < 0.0001), later age of diagnosis of hypertension (34.8 versus 49.7 yr, P = 0.001) and lower prevalence of hypertension at younger ages. Sonographic findings did not differ significantly between both groups. Although anticipation was observed in both groups, it did not affect the majority of families. No signs of imprinting were found in this study, and the only gender effect was an earlier age of onset of ESRD in men than in women (49.5 versus 53.1 yr in PKD1, P < 0.01 and 70.57 versus 73.6 yr in PKD2, P = 0.1). Molecular analysis of autosomal-dominant polycystic kidney disease allows presymptomatic diagnosis in individuals younger than age 30, and helps in establishing prognosis.

We report two brothers with a cystic malformation of the kidneys, liver, and pancreas. In both cases the malformation was fatal and the children died shortly after birth. The pathological findings, consisting of multicystic dysplastic kidneys, dilated and dysgenetic bile ducts, dilated pancreatic ducts, and polysplenia, correspond to those reported by Ivemark as renal-hepatic-pancreatic dysplasia. Many polymalformation syndromes include cystic affectation of these three organs, so this syndrome could be an isolated entity or a final common pathway of response of these organs to a variety of developmental disturbances, which could also include splenic abnormalities. We propose an autosomal recessive pattern of inheritance for renal-hepatic-pancreatic dysplasia.

Background: Hemolytic uremic syndrome (HUS) is a rare, well-characterized complication of the puerperium constituting a cause of acute renal failure after an uncomplicated delivery. However, its presentation during pregnancy is unusual, with only four cases before the 20th week of gestation reported., Case: A case of HUS occurred at 18 weeks' gestation. Despite the initial severity of the clinical presentation, therapy, consisting of corticosteroids and plasmapheresis, resulted in complete recovery of renal function, with a rapid improvement in thrombocytopenia and hemolysis. No remarkable events occurred after this episode, and a full-term pregnancy with a normal fetal outcome was achieved., Conclusion: We are aware of no other reports of successful pregnancy after the development of HUS during the first half of pregnancy. This provides evidence that continuation of pregnancy until term is possible. Rapid diagnosis and intensive treatment with plamapheresis, corticosteroids and dialysis, when required, may improve the maternal and fetal prognosis.

From January 1982 to December 1993, 30 patients with multiple myeloma (MM) required haemodialysis (HD) at our institution. The subgroup of 20 patients who survived more than 2 months on HD is the subject of this study. Four patients were already on HD, due to previous nephropathy, when MM was diagnosed. 13 patients presented with acute renal failure and were on dialysis from the time of diagnosis. The remaining three cases developed renal failure later in the course of the disease. The objective response rate was 40% (8/20). Only two patients could discontinue HD (one had a late partial recovery and one received a kidney graft). Mean hospitalization per year was 19.3 d. The subgroup of patients who survived < 1 year spent a mean of 38.3 d in hospital. Whereas in the subgroup with a survival > 1 year mean hospitalization days was 9.6 (P < 0.001). The median survival was 20 months and six patients survived for > 3 years. In summary, patients with MM and severe renal failure who survive the first 2 months on dialysis have an objective response rate to chemotherapy of 40% and a median survival of almost 2 years, with 30% long-term survivors.

Erythromycin has recently been shown to exert a great effect on gastroduodenal motor activity. This prokinetic action may be clinically useful in patients with gastrointestinal hypomotility such as diabetic or postsurgical gastroparesis. The case of a diabetic patient who underwent antrectomy Billroth II for gastric cancer is presented. Severe gastroparesis appeared after surgery and nasogastric aspiration could not be removed, although the patient was treated with metoclopramide, and glucose levels, hydroelectrolytic balance and nutritional status were corrected. Forty-one days after the first operation, a second gastrectomy with Bilroth II reconstruction was performed because of supposed anastomotic narrowing, which was not confirmed at surgery. Fourteen days later, i.v. erythromycin (200 mg/4h) was started owing to gastroparesis persistence. Six days after treatment the patient tolerated oral ingestion. Prokinetic drugs constitute the specific therapy for gastroparesis. Metoclopramide is the most used, although its efficacy is limited. In the last few years, erythromycin has proved to have a powerful effect on gastroduodenal motility. This effect is mediated, at least in part, by its motilin stimulating activity accelerating gastric emptying. Our patient completely recovered from gastroparesis after erythromycin treatment. Recent results of erythromycin therapy in these patients have been promising, despite the difficult management involved.

Mitomycin C (MMC) is an alkylating agent that has been recently associated with the hemolytic-uremic syndrome (HUS). Pulmonary impairment in the HUS is rarely reported in the literature, and no reports of pulmonary hemorrhage, as a clinical feature of the HUS, have been documented. We describe two women who developed HUS after MMC therapy and presented massive pulmonary bleeding. Pulmonary hemorrhage is an uncommon feature in the HUS, and seems to appear especially in the HUS associated with MMC therapy. It is extremely resistant to standard treatment, and confers a poor prognosis for this disease.

The localized form of Castleman disease is rare and of unknown origin. Pelvic location of the disease is unusual. We report the CT and MR appearance of a case of pelvic Castleman disease presenting with characteristic calcifications and hypervascularity.

Cystic lymphangioma are rare benign tumours which are manifested as unilocular o multilocular masses containing chylous or serous material. Its origin is generally accepted to be a congenital lymphatic inflammation. It is difficult to make a preoperative diagnosis. Occasionally, when these lesions are suspected, they may be diagnosed by lymphography. We describe one case which underwent subtotal gastrectomy. At the microscopic examination dilated lymphatic channels were present with some lymphatic inflammation.